Background: Despite high survival rates in childhood craniopharyngioma, prognosis is frequently impaired due to sequelae. Radical surgery was the treatment of choice for several decades. However, even at experienced surgical facilities radical surgery can result in hypothalamic disorders such as severe obesity.
Objective and hypotheses: We analyzed, whether treatment strategies for childhood craniopharyngioma patients recruited in German studies (KRANIOPHARYNGEOM 2000/2007) have changed during the last 12 years.
Method: We compared the grade of pre-surgical hypothalamic involvement, the treatment, degree of resection and grade of surgical hypothalamic lesions between patients recruited in KRANIOPHARYNGEOM 2000 (n=120; 20012007) and KRANIOPHARYNGEOM 2007 (n=106; 20072012).
Results: The grade of initial hypothalamic involvement was similar in patients treated 20012007 and 20072012. The degree of resection was more radical (P=0.01) in patients recruited 20012007 (38%) when compared with patients treated 20072012 (18%). In patients with pre-surgical involvement of anterior/posterior hypothalamic areas, the rate of hypothalamus-sparing operations resulting in no (further) hypothalamic lesions was higher (P=0.005) in patients treated 20072012 (35%) in comparison with the 20012007 cohort (13%). Event-free-survival rates were similar in both cohorts.
Conclusion: A trend towards less radical surgical approaches is observed, which was accompanied by a reduced rate of severe hypothalamic lesions. Event-free survival was not compromised by this development. Radical surgery is not an appropriate treatment strategy in patients with hypothalamic involvement. Despite previous recommendations to centralize treatment at specialized centers, a trend towards further decentralization was seen. Treatment should be confined to experienced multidisciplinary teams.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology